Dyspnea and Cough DSA

Question 1

Granulomatosis with Polyangiitis (Wegener’s granulomatosis) is noted to have a triad of what 3 features?

Answer 1

URT disease
LRT disease
Glomerulonephritis

Question 2

What is needed for Dx of Polyangiitis with Granulomatosis?

Answer 2

Tissue Bx
ANCAs
Triad

Question 3

ANCAs of Polyangiitis with Granulomatosis are usually directed against…

Answer 3

Proteinase-3

Question 4

What is the general pathologic features of Polyangiitis with Granulomatosis? (3)

Answer 4

Vasculitis of small aa., arterioles, and capillaries
Necrotizing granulomatous lesions of URT and LRT
GN

Question 5

What is the patient demographic of Polyangiitis with Granulomatosis?

Answer 5

4-5th decades, M=F

Annual incidence of 12 per million

Question 6

How long does it take for Polyangiitis with Granulomatosis to develop? What are some early presentations?

Answer 6

4-12 mo. with fever, malaise, weight loss.

Question 7

DZs on DDx of Polyangiitis with Granulomatosis

Answer 7

PAN
Microscopic polyangiitis
Churg-Strauss
Chronic sinusitis
Goodpasture syndrome
SLE
Sarcoidosis
Cocaine

Question 8

What 2 lab tests should be ordered for Polyangiitis with Granulomatosis?

Answer 8

c-ANCA

• Abs to proteinase-3 (from neutrophil granules)
• High specificity (>90%)
• If Dz is active, c-ANCA will be high

p-ANCA

• Abs to myeloperoxidase
• Less specific than c-ANCA
• 10-25% w/ classic Polyangiitis with Granulomatosis have p-ANCA if kidney involvement is present
• Proteinuria
• Hematuria and casts

Question 9

What imaging should be done for Polyangiitis with Granulomatosis?

Answer 9

Chest CT

Question 10

ANCA is not enough to Dx Polyangiitis with Granulomatosis. What else is paramount?

Answer 10

Bx and histology

• vasculitis
• granulomatous inflammation
• acute/chronic inflammation
• renal Bx for crescents

Question 11

What is the treatment for Polyangiitis with Granulomatosis is divided into two phases:

How is the therapy determined?

Answer 11

Induction of remission
Maintenance of remission

Determined on whether the pt. has a mild disease (no kidney involvement) vs. severe life/organ-threatening disease (RPGN, Pulm. hemorrhage, etc)

Question 12

What is the prognosis of Polyangiitis with Granulomatosis without treatment?

What can be given as Tx? Do they work well?

Answer 12

Fatal usually within 1 year.

Cyclophosphamide and Prednisone.
Remissions have been in up to 75%, but half will suffer recurrences.

Question 13

Churg-Strauss is vasculitis of:

What organ systems are affected?

Answer 13

Small and medium aa. in patients with asthmatic sx.

Skin, lung, maybe heart, GI, nerves.

Question 14

Major clinical finding of Churg-Strauss:

What is seen on CXR?

Answer 14

*Peripheral eosinophilia

Lung opacities to nodules

Question 15

What is seen on Bx to make a Dx of Churg-Strauss?

Answer 15

Fibrinoid necrotizing epithelioid and eosinophilic granulomas

Question 16

What is the Tx for Churg-Strauss initially?

What can it be replaced by for maintenance therapy?

Answer 16

Prednisone and Cyclophosphamide

Cyclophosphamide replaced with Methotrexate or Azathioprine

Question 17

Populations with increased risk of Sarcoid?

Answer 17

North America AA (F>M)
Northern European white

Onset in 3-4th decade usually

Question 18

Presenting SX of Sarcoid:

Answer 18

Malaise, fever, DOE
Erythema nodosum, iritis, peripheral neuropathy, arthritis, CM may prompt initial eval.
Can be ASX.
Parotid gland enlargement
HSM, Lymphadenopathy

Question 19

Myocardial sarcoid is found in only 5% of patients, but it can lead to: (3)

Answer 19

Restrictive CM
Cardiac dysrhythmias
Conduction disturbances

Question 20

Lab abnormalities in Sarcoid

Answer 20

Leukopenia
Possible hypercalcemia
Elevated ACE

Question 21

Sarcoid on PFT

Answer 21

Restrictive changes with decreased lung volumes and diffusion capacity most common. May reveal airflow obstruction.

Question 22

What test is positive in 70% of patients with Sarcoid?

Answer 22

Skin test anergy

Question 23

Stage I, II, III and IV Sarcoid on imaging

Answer 23

Stage I - hilar adenopathy
Stage II - hilar adenopathy w/ parenchymal involvement
Stage III - only parenchymal involvement
Stage IV - advanced fibrotic changes in upper lobes

Question 24

What is useful for Dx of Sarcoid?

Answer 24

Bx of non-caseating granulomata
Transbronchial Bx
ECG may show conduction problems and dysrhythmias

Question 25

What is often seen in bronchoalveolar lavage in Sarcoid?

What is it useful for?

Answer 25

Increased lymphocytes with a high CD4/CD8 ration

Used to follow Dz, not for Dx

Question 26

What is given for Tx of Sarcoid?

What immunosuppressives are given?

Answer 26

Prednisone

Methotrexate or Cyclosporine, or anti-TNF w/ Infliximab

Question 27

Demographic of patients with IPF

Answer 27

6-7th decade, M>F

More common in past smokers

Question 28

What genetic variants (mutations) are associated with IPF? (3)

Answer 28

Mutations in surfactant protein C
Telomerase
Polymorphisms of MUC5B

Question 29

Presentation of IPF

Answer 29

**Worsening DOE
Dry cough
Basilar crackles
Clubbing
Cyanosis
RSHF

Question 30

Workup for IPF

Answer 30

CT/CXR
PFT
6 min walk
Labs
**Lung Bx

Question 31

Meds for IPF (2) and their S/E

Answer 31

Pirfenidone - anti-fibrotic med. No known MOA. Taken 3x/day. S/E are nausea, abdominal pain, photosensitivity. Must monitor LFTs.

Nintedanib - tyr kinase inhibitor. S/E are diarrhea. LFTs must be monitored.

Question 32

Aside from the meds, what can be given to patients with IPF?

Answer 32

O2
Pulm rehab
Transplant

Question 33

Risk factors for PE

Answer 33

Immobility
Hyperviscosity
Increased venous pressures
Vessel damage
Hypercoagulative states